What is autoinflammatory disease?

What is autoinflammatory disease?

Autoinflammatory diseases refer to problems with the innate immune system’s reactions. Immune cells target the body’s own healthy tissues by mistake, signaling the body to attack them. This can cause intense episodes of inflammation that result in such symptoms as fever, rash, or joint swelling.

What does MEFV stand for?

MEFV (Mediterranean fever) is a human gene that provides instructions for making a protein called pyrin (also known as marenostrin). Pyrin is produced in certain white blood cells (neutrophils, eosinophils and monocytes) that play a role in inflammation and in fighting infection.

What is MCC950?

MCC950 is a potent and specific inhibitor of the NLRP3 (NOD-like receptor pyrin domain-containing protein 3, cryopyrin, or NALP3) inflammasome [1,2]. The multiprotein NLRP3 inflammasome complex is a key player in innate immunity.

Are autoinflammatory diseases fatal?

Symptoms of autoinflammatory disease Other common symptoms include inflammation of muscles, joints, skin, the gastrointestinal tract and internal organs. If not properly controlled, repeated inflammation can lead to potentially fatal deposits of amyloid protein in vital organs like the kidney.

Can you cure autoinflammatory disease?

Autoimmune disorders in general cannot be cured, but the condition can be controlled in many cases. Historically, treatments include: anti-inflammatory drugs – to reduce inflammation and pain. corticosteroids – to reduce inflammation.

How many inflammasomes are there?

Four key inflammasomes, namely NLRP1, NLRP3, NLRC4, and AIM2, have been best characterized. Activation of these inflammasomes leads to the processing and secretion of inflammatory cytokines, including IL-1β and IL-18, as well as leading to an inflammatory form of cell death termed pyroptosis.

Is pyroptosis regulated cell death?

Pyroptosis: a master regulator of inflammation. Pyroptosis is a type of cell death culminating in the loss of plasma membrane integrity and induced by activation of so-called inflammasome sensors.

Is FMF fatal?

With early and regular treatment, individuals with FMF can live a normal lifespan and may even be free of symptoms. The disease has the potential to be life-threatening if the patient develops kidney failure (which may result when a person is untreated or does not respond to treatment).

What does FMF pain feel like?

Abdominal pain. Chest pain, which can make it hard to breathe deeply. Painful, swollen joints, usually in the knees, ankles and hips. A red rash on your legs, especially below your knees.

Are inflammasomes Nlrs?

Inflammasomes are comprised of homoligomerization of a NLR, procaspase-1, and ASC. Several NLR family members have been demonstrated to form inflammasomes, e.g., NLRP1, NLRP3, and NLRC4. Additionally, as alluded to above, certain non-NLR PRRs can also form inflammasomes and activate IL-1β and IL-18.

What triggers autoinflammatory?

Autoinflammatory diseases are caused by abnormal activation of the innate immune system, leading to recurrent episodes of fever and inflammation that can damage vital organs. Our researchers are revealing how the innate immune system goes awry in autoinflammatory disease.

Can autoimmune cause death?

In the large majority of cases, autoimmune diseases are not fatal, and those living with an autoimmune disease can expect to live a regular lifespan. There are some autoimmune diseases that can be fatal or lead to life-threatening complications, but these diseases are rare.